Evaluative Epidemiology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy.
Cancer Therapy Evaluation Program, National Cancer Institute, Bethesda, MD, USA.
Cancer Med. 2020 Aug;9(15):5632-5642. doi: 10.1002/cam4.3137. Epub 2020 May 21.
Geographical variability of cancer burden was almost exclusively estimated for common cancers. Since rare cancers (RC) have become an area of priority for basic and clinical research and public health organizations, this paper provides, using a common methodology, a detailed comparison of incidence and survival for RC in the US and Europe. We estimated incidence and net survival of 199 malignant RC from data of 2 580 000 patients collected by 18 US-SEER and 94 European registries, diagnosed within the most recent common period 2000-2007. RC were defined according to the criterion of crude annual incidence rates <6/100 000. In total, 196 RC were classified as rare in both populations. Of these, 43 had incidence rates significantly different by at least 0.2 per 100 000:34 higher in the US and 9 higher in Europe. Five-year net survival for all RC combined significantly differed: 54% in the US and 48% in Europe. Survival for 62 RC was significantly higher in the US vs 6 higher in Europe. Differences were not concentrated in a particular cancer family, and were mostly relevant for cases diagnosed >65+ years of age. Use of standardized methods evidenced that incidence and survival rate of majority of RC were higher in the United States compared to Europe. Possible reasons for such differences, requiring further studies, include distribution of risk factors, ability to diagnose RC, different registration practices, and use of updated International Classification of Diseases for Oncology.
癌症负担的地理差异主要是通过对常见癌症进行评估来估计的。由于罕见癌症（RC）已成为基础和临床研究以及公共卫生组织的重点领域，因此本文使用通用方法，详细比较了美国和欧洲罕见癌症的发病率和生存率。我们根据粗年发病率<6/100 000的标准，从美国 18 个 SEER 和 94 个欧洲登记处收集的 258 万患者的数据中估计了 199 种恶性 RC 的发病率和净生存率，这些患者均在最近的共同时间段 2000-2007 内被诊断出患有 RC。总共 196 种 RC 在两个地区均被归类为罕见癌症。其中，有 43 种癌症的发病率至少相差 0.2/100 000：美国高 0.2，欧洲高 0.9。所有 RC 联合的五年净生存率差异显著：美国为 54%，欧洲为 48%。 62 种 RC 的生存率在美国显著高于欧洲，高 6 个百分点。差异并未集中在特定的癌症家族中，而且主要与 65 岁以上诊断出的病例有关。使用标准化方法表明，与欧洲相比，美国大多数 RC 的发病率和生存率较高。需要进一步研究的这种差异的可能原因包括危险因素的分布、诊断 RC 的能力、不同的登记实践以及使用最新的国际肿瘤学疾病分类。
